Rosai-Dorfman Disease: Another Possible IgG4 Sclerosing Disease?
Daniel B Wimmer, Young H Ko, Jooryung Huh, Bong Hee Park, Annisa Lewis, Steven Shen, Alberto G Ayala, Jae Y Ro. The Methodist Hospital, Weill Medical College of Cornell University, Houston, TX; Sam Sung, Seoul, Korea; Asan Medical Center, Seoul, Korea
Background: Rosai-Dorfman disease (RDD) is a rare non-malignant histiocytic proliferation of unknown etiology. IgG4 sclerosing disease is a recently described entity, known to occur in a variety of anatomic sites, and characterized by sclerosis in the presence of an IgG4-positive plasma cell infiltration. Recent reports have examined the possible link between Rosai-Dorfman disease and IgG4 related sclerosing disease. We present 7 cases of RDD that have features of IgG4 related sclerosing disease and attempt to forge a relationship between the two entities.
Design: We reviewed 7 cases of RDD for the characteristic features of IgG4 sclerosis, including fibrosis, obliterative thrombophlebitis, lymphoplasmacytic infiltration, and specifically, the number of IgG4-positive plasma cells. Using immunohistochemistry (IHC) to identify these cells, we adopted a threshold of 30 IgG4-positive cells / HPF for an average of three HPF before ascribing IgG4 sclerosis.
Results: The seven patients' ages ranged from 11 to 70 years old. The ratio of females to males was 5:2. One patient had a history of autoimmune pancreatitis with elevated serum IgG4 levels; findings that have been linked to IgG4 sclerosis. Two patients had multiple lesion sites. Lymph node involvement was documented in two, but extranodal disease existed in all seven patients. Involved sites included the nasal cavity (2), the mediastinum (2), arm (2), skin (1), colon (1), and the subglottic region (1). Presenting symptoms varied from discrete masses and related sequelae to incidental discovery on radiographic imaging.
Six of the seven collected cases met or exceeded our established 30 IgG4 cells / HPF. IHC stains showed three-field-averages ranging from 30 – 146 IgG4-positive cells.
Conclusions: Our data showed a marked increase in IgG4 positive cells in six of seven RDD cases. This may suggest a possible close relationship between RDD and IgG4 sclerosing disease, but the relationship does not appear absolute. Further investigation and larger sample sizes are necessary to evaluate and define the relationship between RDD and IgG4 sclerosis.
Category: Bone & Soft Tissue
Monday, February 28, 2011 9:30 AM
Poster Session I Stowell-Orbison/Surgical Pathology/Autopsy Awards Poster Session # 9, Monday Morning